Abstract
Background: Sickle cell disease (SCD) is associated with a chronic inflammatory state. SCD patients often seek care in the Emergency Department (ED) due to vaso-occlusive crisis (VOC), the most common complication of SCD. Currently, no diagnostic test can determine if a SCD patient is having an acute VOC. Methodology: Irrespective of the clinical diagnosis and type of sickle cell crises the confirmation of haemoglobin pattern in sickle cell disease patients and healthy subjects were done by sickling test, hemoglobin electrophoresis or high performance liquid chromatography.
Results: CRP status in Group I (Hb electrophoretically AA) healthy subjects were analyzed. It showed that irrespective of their age and sex 100% were CRP negative during their healthy state. Diarrhoea (26%), LRTI (16%), URTI (14%) and malaria (12%) were more prevalent infection in sickle cell patients in the present study. It was observed that out of 21 patients with CRP positivity in early phase 17 patients (80.9%) developed vaso-occlusive crisis during their follow up.
Conclusion: The present study demonstrated a strong association between the inflammatory biomarker hs-CRP and vasococclusive crisis in homozygous sickle cell disease (HbSS) and sickle cell-b thalassemia disease (Hb S-b thal). We believe these results highlight the clinical relevance of inflammation in microvessel occlusive complications, and provide a basis for the study of hs-CRP as a potential biomarker for predictive modelling of clinical outcomes in SCD.
Keywords: Sickle Cell Disorder, homozygous sickle cell disease (HbSS), sickle cell-b thalassemia disease, vaso-occlusive crisis, hemoglobin electrophoresis, biomarker, C-reactive protein.
References
- Oluwatoyin Olatundun Ilesanmi. Pathological basis of symptoms and crises in sickle cell disorder: implications for counseling and psychotherapy. Hematol Rep. 2010 Jan 26; 2(1): e2.
- Embury SH, Hebbel RP, Mohandas N, Steinberg MH, editors. Basic Principles and Clinical Picture. New York: Raven Press; 1994. Sickle cell disease; pp. 311–311.
- Benjamin LJ. Nature and treatment of the acute painful episode in sickle cell disease. In: Steinberg MH, et al., editors. Disorders of Hemoglobin: Genetics, Pathophysio-logy, and Clinical Management. Cambridge: 2001. pp. 671–710.
- Benjamin LJ, Payne R. Pain in sickle cell disease: a multidimensional construct. In: Pace B, editor. Renaissance of Sickle cell disease Research in the Genomic Era. London: Imperial College Press; 2007. pp. 99–118.
- Bauer J. Sickle cell disease: pathogenetic, clinical and therapeutic considerations. Arch Surg. 1940; 41:1344–62.
- Emmel VE. A study of erythrocytes in a case of severe anemia with elongated and sickle-shaped red blood corpuscles. Arch Intern Med. 1917; 20:586–98.
- Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004; 364:1343–1360.
- Schimidt RM, Brosius EM. Center for disease control bureau of laboratories haematology division.7th edition. 1975. Basic laboratory methods of hemoglobinopathy detection.
- Junius GA, Martin H. Laboratory detection of hemoglobinopathies and thalassemias, in Hematology Basic Principles and Practice, H. Ronald, B. J. Edward, J. S. Sanford, F. Bruce, and J. C. Harvey, eds., Churchill Livingstone, London, 1991, 1815–1827.
- Richards NP, Elliott TS, Powell RJ, O’Callaghan C, Franklin P. Can the rapid semiquantitative estimation of serum C reactive protein be adapted for the management of bacterial infection? Journal of Clinical Pathology. 1985; 38(4):464-467.
- Vallance H1, Lockitch G. Rapid, semi-quantitative assay of C-reactive protein evaluated. Clin Chem. 1991 Nov; 37(11):1981-2.
- Shukla RN, Solanki BR. Sickle cell trait in Central India. Lancet, 1958; 1(7015):297-298.
- Salim S, Arrayed AI, Haites N. Features of sickle cell disease in Bahrain. Eastern Mediterranean Health Journal 1995; 1(1): 112-119.
- Krishnan S, Setty Y, Betal SG, Vijender V, Rao K, Dampier C, et al. Increased levels of the inflammatory biomarker C-reactive protein at baseline are associated with childhood sickle cell vasocclusive crises. Br J Haematol. 2010 Mar; 148(5): 797–804.
- Gelpi AP. Sickle cell disease in the Middle East. In: Fried W ed. Comparative clinical aspects of sickle cell disease. New York: Elsevier North Holland, 1982; 35–50.
- Singhal A, Doherty JF, Raynes JG, McAdam KP, Thomas PW, Serjeant BE, et al. Is there an acute-phase response in steady-state sickle cell disease? Lancet. 1993 Mar 13; 341(8846):651-3.
- Svarch E, Hernández P, Ballester JM. Sickle Cell Disease in Cuba. Available at http://bioinformatica.uab.es/biocomputacio/treballs00-01/cuaresma/a%20Cuba.htm [Accessed on 12 Sept, 2018]
- Platt OS. Sickle cell anemia as an inflammatory disease. Journal of Clinical Investigation 2000; 106, 337–338.
- Belcher JD, Bryant CJ, Nguyen J, Bowlin PR, Kielbik MC, Bischof JC, Hebbel RP, Vercellotti GM. Transgenic sickle mice have vascular inflammation. Blood. 2003 May 15; 101(10):3953-9. Epub 2003 Jan 23.
- Koenig W, Sund M, Frohlich M, Fischer HG, Lowel H, Doring A, et al. C-Reactive protein, a sensitive marker of inflammation, predicts future risk of coronary heart disease in initially healthy middle-aged men: results from the MONICA (Monitoring Trends and Determinants in Cardiovascular Disease) Augsburg Cohort Study, 1984 to 1992. Circulation 1999; 99: 237-242.
- Ridker PM, Hennekens CH, Buring JE, Rifai N. C-reactive protein and other markers of inflammation in the prediction of cardiovascular disease in women. New England Journal of Medicine 2000; 342: 836–843.
- Verma S, Szmitko PE, Ridker PM. C-reactive protein comes of age. Nature Clinical Practice. Cardiovascular Medicine 2005; 2: 29–36.
Corresponding Author
Dr Sheela Kandulna (Goswami)
Email: This email address is being protected from spambots. You need JavaScript enabled to view it., Mobile: 9434236304